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镰状细胞病的肾脏表现

Author
Edgar V Lerma, MD, FACP, FASN, FAHA
Section Editor
Gary C Curhan, MD, ScD
Deputy Editors
Alice M Sheridan, MD
Jennifer S Tirnauer, MD
Translators
张燕, 主任医师

引言

血管阻塞性现象和溶血是镰状细胞病的临床特点。脱氧血红蛋白S的聚合导致红细胞(red blood cell, RBC)变形为典型的镰刀形以及红细胞的可变形性显著降低，是血管阻塞现象的主要原因[1]。随后发生的红细胞膜结构和功能改变、细胞容积控制障碍，以及对血管内皮的黏附增加，也发挥了重要的作用[1,2]。 (参见“镰状血红蛋白多聚体：结构和功能特性”)

血管阻塞可引起疼痛反复发作和多种严重的可导致终身残疾甚至死亡的器官系统并发症。镰状细胞病的肾脏表现将总结在此。该疾病的其他表现、诊断和治疗，以及其他镰状细胞病综合征将单独讨论。 (参见“镰状细胞病的临床表现概述”和“镰状细胞病的诊断”和“镰状细胞病的治疗和预后概述”和“变异镰状细胞综合征概述”)

流行病学

临床上显著的肾脏受累在镰状细胞病中比在镰状细胞遗传性状或复合异常血红蛋白病中更常见，但肾髓质癌例外，肾髓质癌似乎更常见于镰状细胞遗传性状患者[3-7]。镰状细胞病和镰状细胞遗传性状均可发生肾梗死和肾乳头坏死。放射影像学研究估计其患病率为30%-40%[8,9]。

镰状细胞病患者蛋白尿的患病率估计为20%-25%，5%-30%报道了肾功能下降[10-12]。而据报道，在其他镰状异常血红蛋白病患者中，白蛋白尿和/或蛋白尿的患病率为8%-30%[12-14](随年龄的增长而增加[12])，肾功能下降的发生率为6%[12]。

在美国，镰状细胞病占所有新发终末期肾病(end-stage renal disease, ESRD)病例的不足1%[15]。镰状细胞病患者的肾功能衰竭发病率在两项相关的队列研究中得到了最好说明[4,5]：

                          

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Literature review current through: 2017-06 . | This topic last updated: 2016-11-23.
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